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Collaborator:

National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)

Biliary atresia and idiopathic neonatal hepatitis are the most common causes of jaundice and hyperbilirubinemia that continue beyond the newborn period. The long term goal of the Biliary Atresia Research Consortium (BARC) is to establish a database of clinical information and serum and tissue samples from children with biliary atresia (BA) and idiopathic neonatal hepatitis (INH) to facilitate research and to perform clinical, epidemiological and therapeutic trials in these two important pediatric liver diseases.

Infants presenting with cholestasis who are diagnosed with biliary atresia.

Non-Biliary Atresia

Infants presenting with cholestasis without a diagnosis of biliary atresia.

Detailed Description:

This is a multi-center project to establish a prospective database of clinical information and a repository of blood and tissue samples from children with diagnoses of neonatal liver diseases, such as biliary atresia and neonatal hepatitis, in order to perform research in these important liver problems. Children will be screened and enrolled at presentation at the participating pediatric liver sites. Subjects diagnosed with biliary atresia will be followed intensively for the first year, at 18 months of age, and then annually up to 15 years of age. Other subjects diagnosed with cholestasis will be followed on the same schedule; if there is complete (clinical and biochemical) resolution of their underlying liver disease off all therapy, there will be one follow up visit within one year (preferably scheduled at the time of the next planned follow up visit or at 12 months of age, whichever is later) for data collection and to obtain blood samples. The development of a serum and tissue bank of specimens from children with various neonatal cholestatic disorders will be an invaluable tool for current and future investigations into the etiology and pathogenesis of hepatobiliary injury in the infant.

Detailed clinical data, laboratory investigations, liver biopsy specimens, and long-term follow-up of outcomes are part of the normal standard of care with respect to the diagnosis and treatment of the subjects with liver problems. This research involves the collection of diagnostic, clinical and outcome data concerning the subject, which is kept without identification (coded) in a national research database of infants with liver disease. Samples of blood and urine will be obtained for later research analysis, whenever possible, at the time of clinically indicated blood draws or when there is IV access for a clinical procedure. When liver biopsy specimens are obtained for diagnostic purposes, any liver biopsy specimen in excess of that needed for diagnostic use will be sent to the tissue repository. When a portoenterostomy or liver transplant occurs, sections of the liver, biliary remnant and bile specimens, if removed in the course of surgery and in excess of that needed for diagnostic use, will be sent for the repository. These specimens will be used in investigations into the mechanisms and causes of the liver damage that occur in the subject's condition. As part of the standard of care, the study will follow-up and record progress of the liver problem by routine clinical examinations and laboratory tests for up to 15 years. All data from this study will be kept in a secure research database at the data coordinating center.

Eligibility

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Ages Eligible for Study:

up to 6 Months (Child)

Sexes Eligible for Study:

All

Accepts Healthy Volunteers:

No

Sampling Method:

Non-Probability Sample

Study Population

This study population will be selected from the patient base of participating speciality care clinics.

Criteria

INCLUSION CRITERIA

Infant's age less than or equal to 180 days at initial presentation at the ChiLDREN clinical site.

Diagnosis of cholestasis defined by serum direct or conjugated bilirubin greater than 20% of total and greater than or equal to 2 mg/dl.

The subject's parent(s)/guardian(s) willing to provide informed written consent.

EXCLUSION CRITERIA

Acute liver failure.

Previous hepatobiliary surgery with dissection or excision of biliary tissue.

Diagnoses of bacterial or fungal sepsis (except where associated with metabolic liver disease)

Diagnoses of hypoxia, shock or ischemic hepatopathy within the past two weeks (If the cholestasis persists beyond two weeks of the initiating event, the infant can be enrolled).

Diagnosis of any malignancy.

Presence of any primary hemolytic disease (except when diagnosed with biliary atresia or another cholestatic disease being studied by ChiLDREN).

Diagnosis of any drug or TPN-associated cholestasis (except when diagnosed with biliary atresia or another cholestatic disease being studied by ChiLDREN).

Diagnosis with ECMO-associated cholestasis.

Birth weight less than 1500g (except when diagnosed with biliary atresia).

Contacts and Locations

Information from the National Library of Medicine

To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.

Please refer to this study by its ClinicalTrials.gov identifier (NCT number): NCT00061828